Syndrome Cudgel Johnson

Description:

Syndrome the Cudgel Johnson - the chronic benign disease which is shown non-constant jaundice with increase in level of preferential conjugated bilirubin and a biliribinuriya. It is inherited on autosomal recessively type, is widespread preferential on the Middle East among the Iranian Jews.

Syndrome reasons Cudgel Johnson:

At the heart of Dabin-Johnson's syndrome (family chronic idiopathic jaundice with not identified pigment in hepatic cells) inborn defect of disturbance of excretory function of hepatocytes (post-microsomal hepatocellular jaundice) lies.

Syndrome symptoms Cudgel Johnson:

At this syndrome bilirubin excretion from hepatocytes is broken that leads to regurgitation of the quoted bilirubin in blood (the conjugated hyperbilirubinemia develops). Along with defect of excretion of bilirubin disturbance of excretion of a bromsulfalein, Bengalese pink, holetsistografichesky means is noted. Excretion of bile acids is not broken.

Adjournment peribiliarno in cytoplasm of hepatocytes of a significant amount of a pigment of dark brown color is characteristic of Dabin-Johnson's syndrome. Macroscopically the liver has color from bluish-green to almost black. The nature of a pigment is not finalized. A. F. Blyuger (1984) assumes that its basis is melanin.

The disease can be shown for the first time by jaundice at pregnancy or against the background of reception of oral contraceptives (both of these states cause deterioration in excretory function of a liver). The forecast is favorable.

The following manifestations are characteristic of Dabin-Johnson's syndrome:
the disease is transmitted in the autosomal and dominant way;
more often men are ill;
the first clinical signs of a disease are found during the period from the birth up to 25 years;
the main symptom of a disease - the chronic or intermittent jaundice which is unsharply expressed;
jaundice is followed by fatigue, nausea, a loss of appetite, sometimes abdominal pains; seldom - not intensive skin itch;
content in blood of bilirubin is increased to 20-50 µmol/l (seldom to 80-90 µmol/l) due to preferential increase in the conjugated (straight line) of fraction;
the bilirubinurea is observed; dark color of urine;
the liver at most of patients is not increased, its increase by 1-2 cm is occasionally noted;
bromsulfaleinovy test, and also radio isotope gepatografiya reveal sharp disturbance of excretory function of a liver; other functional trials of a liver are significantly not changed;
at X-ray inspection of biliary tract lack of contrasting is characteristic or also weak filling of bilious ways and a bubble with a contrast agent is later;
there are no changes of indicators of blood;
the syndrome of a cytolysis of hepatocytes is not characteristic.

Treatment of a syndrome Cudgel Johnson:

The aspiration to avoid provocative factors (an infection, exercise and mental stresses, alcohol intake and gepatotoksichny drugs)
Insolation is contraindicated
Diet with restriction of the refractory fats and products containing preservatives. Group B vitamins.
Cholagogue means are recommended.
Sanitation of the chronic centers of an infection and treatment of the available pathology of biliary tract.

Criteria of efficiency of treatment:

Reduction of intensity or elimination of jaundice. Normalization (reliable reduction) of bilirubin level in blood. Treatment duration — during all life.